CNS Embryonal Tumor with PLAGL Amplification, a New Tumor in Children and Adolescents: Insights from a Comprehensive MRI Analysis

A. Tietze, B. Bison, J. Engelhardt, T. Fenouil, D. Figarella-Branger, E. Goebell, J. Hakumäki, E. Koscielniak, L.E. Ludlow, D. Meyronet, P. Nyman, I. Øra, J. Pesola, T. Rauramaa, R.E. Reddingius, D. Samuel, A. Sexton-Oates, A. Vasiljevic, A.K. Wefers, J. Zamecnik, D.T.W. Jones, M.K. Keck, K. von Hoff and European Society for Paediatric Oncology (SIOPE)-Brain Tumour Group

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Abstract

BACKGROUND AND PURPOSE: CNS embryonal tumor with pleomorphic adenoma gene-like 1 (PLAGL1)/pleomorphic adenoma gene-like 2 (PLAGL2) amplification (ET, PLAGL) is a newly identified, highly malignant pediatric tumor. Systematic MRI descriptions of ET, PLAGL are currently lacking.

MATERIALS AND METHODS: MRI data from 19 treatment-naïve patients with confirmed ET, PLAGL were analyzed. Evaluation focused on anatomic involvement, tumor localization, MRI signal characteristics, DWI behavior, and the presence of necrosis and hemorrhage. Descriptive statistics (median, interquartile range, percentage) were assessed.

RESULTS: Ten patients had PLAGL1 and nine had PLAGL2 amplifications. The solid components of the tumors were often multinodular with heterogeneous enhancement (mild to intermediate in 47% and intermediate to strong in 47% of cases). Nonsolid components included cysts in 47% and necrosis in 84% of the cases. The tumors showed heterogeneous T2WI hyper- and isointensity (74%), relatively little diffusion restriction (ADC values less than contralateral normal-appearing WM in 36% of cases with available DWI), and tendencies toward hemorrhage/calcification (42%). No reliable distinction was found between PLAGL1- and PLAGL2-amplified tumors or compared with other embryonal CNS tumors.

CONCLUSIONS: The study contributes to understanding the imaging characteristics of ET, PLAGL. It underscores the need for collaboration in studying rare pediatric tumors and advocates the use of harmonized imaging protocols for better characterization.

ABBREVIATIONS:

ATRT
atypical teratoid/rhabdoid tumor
ETMR
embryonal tumor with multilayered rosettes
ET, PLAGL
CNS embryonal tumor with PLAGL amplification
EVD
external ventricular drain
IQR
interquartile range
PLAGL1
pleomorphic adenoma gene-like 1
PLAGL2
pleomorphic adenoma gene-like 2
pCASL
pseudocontinuous arterial spin-labeling
WHO
World Health Organization

Footnotes

  • M.K. Keck and K. von Hoff contributed equally to this work.

  • This work was funded by the following: B. Bison: German Childhood Cancer Foundation (DKS 2023.10); P.D. Johann: Max-Eder scholarship, German Cancer Aid; T. Perwein: Styrian Children’s Cancer Aid. A. Tietze: German Research Foundation (DFG, SFB295RETUNE). M. Zapotocky: Ministry of Health of the Czech Republic, grant Nr. NU23-08-00460.

  • Disclosure forms provided by the authors are available with the full text and PDF of this article at www.ajnr.org.

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